先天性胆管囊肿癌变的诊断与治疗

目的探讨先天性胆管囊肿癌变的诊断与治疗方法。方法回顾性分析1993年—2004年解放军总医院收治的185例先天性胆管囊肿患者的临床及病理资料。结果185例中合并癌变27例,癌变率为14.6%。先天性胆管囊肿癌变与年龄密切相关(P<0.001),各年龄段癌变率分别为:0～9岁为0%,10～19岁5.1%,20～29岁9.1%,30～39岁16.2%,40～49岁26.7%,50～59岁33.3%,≥60岁50%。6例有胆肠内引流手术史。临床主要表现为上腹疼痛、发热、黄疸和体重下降。诊断方法包括腹部超声、CT、MRI和经内镜逆行胰胆管造影,术前确诊20例,但早期诊断困难。获得根治性切除9例(33.3%)。结论先天性胆管囊肿是一种癌前病变,随年龄增长癌变率急剧增加。儿童期实施完全性囊肿切除是预防胆管囊肿癌变的最有效方法。胆管囊肿癌变病例预后不佳。

Experience of diagnosis and treatment of carcinoma arising from congenital biliary duct cyst

OBJECTIVE: To summarize the experience in diagnosis, prevention and treatment of carcinoma arising from congenital biliary duct cyst. METHODS: The clinical and pathological data of 185 patients with congenital biliary duct cyst admitted to Chinese PLA General Hospital were analyzed retrospectively. RESULTS: Among 185 patients, twenty-seven cases had carcinomas arising from congenital biliary duct cyst, and the frequency of malignant transformation was 14.6%, which closely related to the age (P < 0.001). The incidences of malignancy for different age groups were: 0 for 0-9 age group, 5.1% for 0-19, 9.1% for 20-29, 16.2% for 30-39, 26.7% for 40-49, 33.3% for 50-59, and 50% for over 60, respectively. Six patients had the history of cyst-enterostomy. Abdominal pain, fever, jaundice and weight loss were the main clinical manifestations. Abdominal ultrasonography, CT, MRI or magnetic resonance cholangiopancreatography, MRCP and endoscopic retrograde choledochopancreatography (ERCP) were the main diagnostic methods. For twenty patients (74.1%), a definite diagnosis was made preoperatively, but it's hard to make an early diagnosis. Nine patients (33.3%) underwent curative resection. CONCLUSIONS: Congenital biliary duct cyst is a premalignant lesion, and the incidence of carcinogenesis increases remarkably with age. The most effective method for prevention of carcinogenesis in choledochal cyst is complete excision of choledochal cyst during childhood, and the prognosis is poor for patients with biliary malignancy.